Schneider W, Ditscherlein G
Zentralbl Allg Pathol. 1986;132(5-6):459-70.
A glomerulonephritis (GN) of the basement membrane antibody-mediated type was diagnosed in 51 patients (44 men, 7 women) on the basis of immunohistological findings in renal biopsy material in a period of 15 years. This represented about 5% of the cases in which immunohistologic studies gave evidence of glomerular disease. Young men, about 20 years old, were especially affected: 35 patients were men between ages 16 and 25 (69%). In about half the cases there were also pulmonary manifestations (Goodpasture's syndrome). In 63% of the cases there was extracapillary GN with compression and/or marked destruction of the glomerular tuft. Anti-glomerular basement membrane antibody-mediated nephritis was the most frequent cause of a rapidly progressive extracapillary GN in our sample. The broad use of renal biopsy in Goodpasture's syndrome has demonstrated that the morphologic spectrum of renal manifestations is much wider than originally believed. It ranges from normal or minimal findings and mild mesangioproliferative or focal extracapillary GN to severe diffuse extracapillary GN. The rapidly progressive form of extracapillary GN was not significantly more frequent in Goodpasture's syndrome than in the cases of GN without lung findings (62%). Immunohistologic findings apparently can persist unchanged for months and therefore microscopic examinations do not allow reliable conclusions concerning the natural history of immunologic activity in this process to be drawn.
在15年期间,根据肾活检材料的免疫组织学检查结果,确诊了51例(44例男性,7例女性)基底膜抗体介导型肾小球肾炎(GN)。这约占免疫组织学研究显示有肾小球疾病病例的5%。约20岁的年轻男性受影响尤为严重:35例患者年龄在16至25岁之间(占69%)。约半数病例还伴有肺部表现(Goodpasture综合征)。63%的病例为毛细血管外肾小球肾炎,伴有肾小球毛细血管襻受压和/或明显破坏。在我们的样本中,抗肾小球基底膜抗体介导的肾炎是快速进展性毛细血管外肾小球肾炎最常见的病因。在Goodpasture综合征中广泛应用肾活检已表明,肾脏表现的形态学谱比原先认为的要广泛得多。其范围从正常或轻微表现、轻度系膜增生性或局灶性毛细血管外肾小球肾炎到严重的弥漫性毛细血管外肾小球肾炎。Goodpasture综合征中快速进展型毛细血管外肾小球肾炎的发生率并不比无肺部表现的肾小球肾炎病例显著更高(62%)。免疫组织学检查结果显然可在数月内保持不变,因此显微镜检查无法就该过程中免疫活性的自然病程得出可靠结论。